PubMed Health⌕ Search
Home/Archive/PubMed

PubMed · 16304391

Aggressive peripheral T-cell lymphomas (specified and unspecified types).

Kerry J Savage
View original source ↗Export metadata ↓

Abstract

Peripheral T-cell lymphomas (PTCLs) are a biologically diverse and uncommon group of diseases. Compared to their B-cell counterparts, PTCLs remain largely unexplored and the optimal treatment ill-defined due to disease rarity and biological heterogeneity. For the majority of PTCL subtypes, prognosis is poor with a 5-year overall survival of approximately 30% in most series.The notable exception is ALK-positive anaplastic large-cell lymphoma (ALK-pos ALCL), which has a superior outcome. The international prognostic index can be used to some extent to define risk groups within some PTCL subtypes, including PTCL unspecified (PTCLUS). It is likely that the observed clinical heterogeneity reflects differences at the molecular level. With the more widespread availability of gene expression profiling, it may be possible in the future to further refine the classification of PTCLs and elucidate novel therapeutic targets. Future clinical trials are needed that focus specifically on PTCL to advance our understanding and define the optimal management in this disease.

Explore related subjects

Human T-lymphotropic virus 1HumansKiller Cells, NaturalLeukemia-Lymphoma, Adult T-CellLymphoma, Large B-Cell, DiffuseLymphoma, T-CellLymphoma, T-Cell, PeripheralPrognosisSurvival AnalysisSurvival RateT-LymphocytesTreatment OutcomeJournal Article

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

Kerry J Savage. 2005. Aggressive peripheral T-cell lymphomas (specified and unspecified types).. https://doi.org/10.1182/asheducation-2005.1.267

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed2025

Role of the CTCF binding site in Human T-Cell Leukemia Virus-1 pathogenesis.

During HTLV-1 infection, the virus integrates into the host cell genome as a provirus with a single CCCTC binding protein (CTCF) binding site (vCTCF-BS), which acts as an insulator between transcriptionally active and inactive regions. Previous studies have shown that the vCTCF-BS is important for maintenance of chromatin structure, regulation of viral expression, and DNA and histone methylation. Here, we show that the vCTCF-BS also regulates viral infection and pathogenesis in vivo in a humanized (Hu) mouse model of adult T-cell leukemia/lymphoma. Three cell lines were used to initiate infection of the Hu-mice, i) HTLV-1-WT which carries an intact HTLV-1 provirus genome, ii) HTLV-1-CTCF, which contains a provirus with a mutated vCTCF-BS which abolishes CTCF binding, and a stop codon immediately upstream of the mutated vCTCF-BS which deletes the last 23 amino acids of the p12 gene, and iii) HTLV-1-p12stop that contains the intact vCTCF-BS, but retains the same stop codon in p12 as in the HTLV-1-CTCF cell line. Hu-mice were infected with mitomycin-treated or irradiated HTLV-1 producing cell lines. There was a delay in pathogenicity when Hu-mice were infected with the HTLV-1-CTCF virus compared to mice infected with either HTLV-1-p12 stop or HTLV-1-WT virus. Proviral load (PVL), spleen weights, and CD4 T cell counts were significantly lower in HTLV-1-CTCF infected mice compared to HTLV-1-p12stop infected mice. Furthermore, we found a direct correlation between the PVL in peripheral blood and death of HTLV-1-CTCF infected mice. In cell lines, we found that the vCTCF-BS regulates Tax expression in a time-dependent manner. The scRNAseq analysis of splenocytes from infected mice suggests that the vCTCF-BS plays an important role in activation and expansion of T lymphocytes in vivo. Overall, these findings indicate that the vCTCF-BS regulates Tax expression, proviral load, and HTLV pathogenicity in vivo.

Human T-lymphotropic virus 1↗
PubMed2006

[A case of human T lymphotropic virus type I-related lung disease in which Legionella pneumonia led to the diagnosis].

A 46-year-old man whose parents were from Nagasaki had a 24-hour bath system in his house. He had had a cough for 26 years and dyspnea for 2 years. He consulted our hospital because of the increase of his cough and dyspnea. Chest X ray and CT showed abnormal shadows in both lung fields and Legionella pneumophila type 3 was detected by sputum cultivation. The condition which appeared this time responded to antibiotic medication. However, the abnormal shadows in both lung fields and the abnormalities in respiratory function remained. The remaining abnormal shadows in both lung fields were considered to be the cause of his dyspnea for 2 years and cough for 26 years. Atypical lymphocytes with a floriform nucleus were observed in peripheral blood. Gene analysis detected monoclonal human T lymphotropic virus type I (HTLV-I) provirus DNA. We diagnosed as smoldering type adult-T-cell-leukemia (ATL). Thoracoscopic lung biopsy revealed fibrotic thickening of the interstitial tissue accompanied by structural destruction. The pathological changes in both lung fields were diagnosed as HTLV-I related lung disease and infiltration of ATL. Known pathogens of lung infection accompanying ATL include viruses, acid fast organisms, and fungi. Legionella pneumonia happened to be the opportunity leading to the diagnosis of HTLV-I related lung disease is this case.

Human T-lymphotropic virus 1↗
PubMed2005

Current views in HTLV-I-associated adult T-cell leukemia/lymphoma.

Epidemiological studies have demonstrated that the relative percentage of malignant lymphoid proliferations varies widely according to geographical location and ethnic populations. HTLV-I is the etiological agent of adult T-cell leukemia/lymphoma (ATLL) and is also associated with cutaneous T-cell lymphoma (CTCL). However, a definite role of HTLV-I in mycosis fungoides (MF) and/or Sezary syndrome (SS) remains controversial. While most HTLV-I-infected individuals remain asymptomatic carriers, 1-5% will develop ATLL, an invariably fatal expansion of virus-infected CD4+ T cells. This low incidence and the long latency period preceding occurrence of the disease suggest that additional factors are involved in development of ATLL. In this review, diagnosis, clinical features, and molecular pathogenesis of HTLV-I are discussed.

Human T-lymphotropic virus 1↗